Pediatric Heart Expert Forum
My 6 yr old son has Ventricular Inversion with Corrected TGA
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My 6 yr old son has Ventricular Inversion with Corrected TGA

My 6 yr old son who is a twin has what your hospital calls CCTGA, but our hospital here in AR calls it Ventricular Inversion with corrected TGA plus he has Ebstein's as well. They told us the other day that he needed surgery and that we had to do a heart cath and an MRI to see how big his bottom two chambers are and to measure the pressure. Then we would meet to see if we should do the band surgery or be send to Philadelphia Children's Hospital for the double switch operation. We found these defects when he was 3 weeks old. He also has one pulmonary vein going to the wrong side of the heart. What I would like to know is should we wait til the end of July for these tests or should we see about coming up there for yall to look at him? This is all new to me and I'm very scared for my child. Any advice on this heart defects would be great to try and calm my nerves! Thank you for your time.

Laci, from Arkansas
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Corrected transposition of the great arteries with Ebstein's anomaly is a complex form of congenital heart disease and its management is somewhat controversial.  Generally, the doctors feel that surgery is indicated when the amount of tricuspid valve leakage from the Ebstein's valve becomes severe.  A double -switch operation cannot be done in a 6 year old without a preliminary operation to "prepare" the right sided left ventricle to handle the high pressure work load of the body. That is the role for the pulmonary artery banding.  It takes quite a while (months) to accomplish the goal of increasing the pressure in the left ventricle.  That surgery can be complicated by poor functioning of the left ventricle after the banding.  The cardiac cath is a good idea to know the current pressure in the left ventricle.  Certainly getting the cardiac catheterization in your home state is appropriate and the timing of this summer does not seem problematic unless your child is having significant symptoms that would merit an immediate intervention.  The pulmonary artery banding will need to be done somewhere, unless the LV pressure is already high (which seems unlikely).  Your cardiologist can certainly send the data to CHOP for our review.
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